Glantsman's thrombasthenia

Description:

Glantsman's thrombasthenia – the hereditary disease from group of hemorrhagic diathesis which is characterized by insufficiency of a number of enzymes in thrombocytes, secondary disturbance of retraction of a blood clot and often extended bleeding time at the normal or slightly lowered quantity of thrombocytes, existence of huge thrombocytes; allocate forms with an autosomal and recessive and autosomal and dominant mode of inheritance.

Symptoms of the Thrombasthenia of Glantsman:

Developing of hematomas, bleedings from mucous and hypermenorrheas, and also heavy bleedings in response to normal incentives is characteristic of Glantsman's thrombasthenia (for example, surgical intervention or an injury). The mode of inheritance is preferential autosomal and recessive; the disease with an identical frequency occurs among persons of both sexes; Glantsman's thrombasthenia is registered more often among populations in which closely related marriages are widespread. Clinical manifestations are observed since the birth though the current can be symptomless to an injury or surgery that masks existence of a disease. At heterozygotes disease symptoms usually are absent, but at them the tendency to the raised bleeding can be observed. The acquired form of a thrombasthenia of Glantsman sometimes is associated with the malignant general diseases, uraemia and diseases which are followed by formation of autoantibodies.

Reasons of the Thrombasthenia of Glantsman:

There are 3 types of a thrombasthenia of Glantsman:

•    type 1: heavy deficit of the GPIIb-IIIa complex (<5% нормы);
•    type 2: moderate deficit of the GPIIb-IIIa complex (10-20% of norm);
•    type 3: the option at which the GPIIb-IIIa complex is present at normal or almost normal quantity, but is functionally insolvent.

Nevertheless, correlation between the number of GPIIb-IIIa on a surface of thrombocytes and weight of clinical displays of a disease does not exist.

Treatment of the Thrombasthenia of Glantsman:

Standard treatment of bleedings at patients with Glantsman's thrombasthenia, and also prevention and stopping of bleedings during operations, are transfusions of a trombokontsentrat. However it can lead to emergence of antibodies to GPIIb-IIIa and/or HLA. As a result of it transfusions of a trombokontsentrat stop being an effective method of treatment, the refrakternost develops. Other methods of a stop of slight bleedings are the compression, use of a gelatinous sponge or gauze and anti-fibrinolitic means, such as traneksamovy acid or thrombin locally.